著明な下肢変形を伴う片肢性骨端異形成症の長期治療経過例

A Long-Term Follow up of Dysplasia Epiphysealis Hemimelica with Severe Limb Deformity. Case Report

　われわれは，severe formの片肢性骨端異形成症の男児を4カ月時から15歳現在まで治療中である．幼少時から左下肢の著明な外反膝変形，過成長，また内反尖足変形を認め，多数回手術を行っている．関節外操作による変形矯正を行った膝関節は骨端部がリモデリングされ，関節内操作を行った足関節は強直した．本症の手術適応，術式選択については，病勢を考慮して慎重に決定しなければならない．変形矯正には関節内切除手術を避け，関節外操作を行うことが得策と考える．

　We report the case of a patient with a severe form of dysplasia epiphysealis hemimelica complicated by valgus knee deformity, increased leg length, and equinovarus foot deformity.　We have followed the patient's course from 4 months of age to 15 years of age and performed multiple surgical procedures to treat him.　Surgery on the knee was performed by an extra-articular approach and included osteotomy and stapling of the physis, and finally the epiphysis was remodeled well.　Surgery on the ankle was performed by intra-articular approach because of limited joint motion, and the ankle was fused.　We recommend an extra-articular approach for the treatment of dysplasia epiphysealis hemimelica.　An intra-articular approach is less useful because of the risk of degenerative joint damage.